Biphenotypic sinonasal sarcoma with PAX3-FOXO1 fusion gene! (?)

In our most recent publication in Genes, Chromosomes and Cancer, Alexandra and Waihay have characterized an interesting biphenotypic sinonasal sarcoma with a PAX3-FOXO1 fusion gene. We found the PAX3-FOXO1 genetic fusion characteristic of a highly aggressive, lethal sarcoma (alveolar rhabdomyosarcoma) driving a locally-aggressive, but essentially non-metastatic tumor; how’s that for cellular/genetic context and tumor microenvironment modulating oncogenic events? Our modest case report contributes to an increasing understanding of the genetics of biphenotypic sinonasal sarcoma, and raises interesting biological questions that are also being explored by colleagues from Mayo Clinic and MSKCC in larger case series.